Kidney tumor
General
WHO classification
Topic Completed: 1 December 2016
Minor changes: 28 April 2021
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed search: WHO classification[TIAB] (kidney tumors[TIAB] OR renal tumors[TIAB])
Cite this page: Andeen NK, Tretiakova M. WHO classification. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumorWHOclass.html. Accessed December 2nd, 2021.
Definition / general
- This topic is based on the 2016 World Health Organization classification (Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016)
- We currently plan to update this topic at the end of 2021 based on the new WHO 5th edition
- Based on cytologic, architectural, immunohistochemical, cytogenetic and molecular characteristics
- New entities continue to be described based on immunohistochemical and molecular features - some not yet recognized in WHO Classification
- Use of "granular cell variant" not recommended - may falsely combine a spectrum of tumors with varying clinical significance / treatment: clear cell, chromophobe, unclassified renal cell carcinoma or oncocytoma (Urology 2007;69:452)
- Diagnosis of "sarcomatoid carcinoma" not recommended; classify as high grade carcinoma of type from which it originated (or unclassified) with sarcomatoid features and percentage of sarcomatoid features (Cancer 1997;80:987)
WHO (2016)
Renal cell tumors
Metanephric tumors
Nephroblastic and cystic tumors occurring mainly in children
Mesenchymal tumors
Mesenchymal tumors occurring mainly in children
Mesenchymal tumors occurring mainly in adults
Mixed epithelial and stromal tumor family
Neuroendocrine tumors
Renal hematopoietic neoplasms
Germ cell tumors
Metastatic tumors
Tumor-like lesions
Described entities not currently in WHO
- Clear cell renal cell carcinoma
- Multilocular cystic renal neoplasm of low malignant potential
- Papillary renal cell carcinoma
- Hereditary leiomyomatosis and renal cell carcinoma associated renal cell carcinoma
- Chromophobe renal cell carcinoma
- Collecting duct carcinoma
- Renal medullary carcinoma
- MiT family translocation renal cell carcinomas
- Succinate dehydrogenase deficient renal cell carcinoma
- Mucinous tubular and spindle cell carcinoma
- Tubulocystic renal cell carcinoma
- Acquired cystic disease associated renal cell carcinoma
- Clear cell papillary renal cell carcinoma
- Renal cell carcinoma, unclassified
- Papillary adenoma
- Oncocytoma
Metanephric tumors
- Metanephric adenoma
- Metanephric adenofibroma
- Metanephric stromal tumor
Nephroblastic and cystic tumors occurring mainly in children
- Nephrogenic rests
- Nephroblastoma
- Cystic partially differentiated nephroblastoma
- Pediatric cystic nephroma
Mesenchymal tumors
Mesenchymal tumors occurring mainly in children
- Clear cell sarcoma
- Rhabdoid tumor
- Congenital mesoblastic nephroma
- Ossifying renal tumor of infancy
Mesenchymal tumors occurring mainly in adults
- Leiomyosarcoma (including renal vein leiomyosarcoma)
- Angiosarcoma
- Rhabdomyosarcoma
- Osteosarcoma
- Synovial sarcoma
- Ewing sarcoma
- Angiomyolipoma
- Epithelioid angiomyolipoma
- Leiomyoma
- Hemangioma
- Lymphangioma
- Hemangioblastoma
- Juxtaglomerular cell tumor
- Renomedullary interstitial cell tumor
- Schwannoma
- Solitary fibrous tumor
Mixed epithelial and stromal tumor family
- Adult cystic nephroma
- Mixed epithelial and stromal tumor
Neuroendocrine tumors
- Well differentiated neuroendocrine tumor
- Large cell neuroendocrine carcinoma
- Small cell neuroendocrine carcinoma
- Paraganglioma
Renal hematopoietic neoplasms
Germ cell tumors
Metastatic tumors
Tumor-like lesions
- Xanthogranulomatous pyelonephritis
- IgG4 related disease
Described entities not currently in WHO
- Thyroid-like follicular carcinoma of kidney (Am J Surg Pathol 2009;33:393, Am J Clin Pathol 2015;144:796)
- Transcription elongation factor B (TCEB1) mutated renal cell carcinoma (Mod Pathol 2015;28:845)
- Renal cell carcinoma with smooth muscle stroma (Mod Pathol 2014;27:765, Ann Diagn Pathol 2014;18:291, Ann Diagn Pathol 2016;21:59)
- Renal cell carcinoma with anaplastic lymphoma kinase (ALK) translocation (Cancer 2012;118:4427, Am J Surg Pathol 2016;40:761, Korean J Pathol 2013;47:452, Mod Pathol 2012;25:1516)
- Renal cell carcinoma occurring in patients with prior neuroblastoma (Am J Surg Pathol 2016;40:989)
- Eosinophilic, solid and cystic renal cell carcinoma (Am J Surg Pathol 2016;40:60)
- Biphasic squamoid alveolar renal cell carcinoma (Am J Surg Pathol 2016;40:664)
- Chromophobe renal cell carcinoma with neuroendocrine and neuroendocrine-like features (Ann Diagn Pathol 2015;19:261)
